Disorders of motor neurons include amyotrophic lateral sclerosis (ALS), primary lateral sclerosis (PLS), spinobulbar muscular atrophy (SBMA, or Kennedy’s disease), spinal muscular atrophy (SMA), poliomyelitis, and monomelic amyotrophy (Hirayama disease). Although the pathology, genetics, and clinical...
Motor neuron disorders: Causes, symptoms, risk factors, diagnosis and treatmentsMotoneuronssporadic motor diseasesvirusesgenetic factorsKENNEDYS DISEASEMOUSE MODELTHERAPYIn the Central Nervous System, there are different types of neurons. One of them is motor cells or motor neurons. These cells are of ...
Following disease progression in motor neuron disorders with 3 MUNE methods: MUNE in ALSALS Functional Rating Scaleamyotrophic lateral sclerosisfollow-up studyMScan, motor unit lossMUNE, motor unit number estimationMUNIX, multiple-point stimulation...
determinant of neuronal responsiveness to incoming stimuli, which can condition specific neuronal subpopulations to become differentially vulnerable in neurodegenerative disorders1,2,3,4.
FUS loss-of-function and toxic gain-of-function mechanisms have been proposed to explain how mutant FUS leads to motor neuron degeneration, but neither has been firmly established in the pathogenesis of ALS. Here we characterize a series of transgenic FUS mouse lines that manifest progressive, ...
BackgroundSeveral conditions have been reported to mimic motor neuron disease (MND), and misdiagnosis remains a common clinical problem.ObjectiveTo report
(Lee and Aronson1974; Butterworth and Hicks1977; Forssberg and Nashner1982; Wann et al.1998; Rinaldi et al.2009; Polastri and Barela2013). Using portable devices, a few studies explored the motor signatures of developmental disorders, such as autism (Jansiewicz et al.2006; Anzulewicz et...
Historically, the term “motor neuron diseases” (MNDs) refers to disorders caused by the degeneration of motor neurons (MNs) located at anterior horn in the spinal cord or at the brainstem motor nuclei, with secondary Wallerian degeneration of motor axons (Desai and Olney, 2009). In fact, ...
The three-point difference was chosen based on the clinical judgment of expert consultants in movement disorders and modeling of PPMI data, which were used to model disease progression in the placebo arm of the PASADENA study10. Statistical analyses All the analyses were performed in the mITT ...
The spatial maps of motor neuron activity corresponding to the time at which each hotspot reached a maximum (centre) are laid over the schematics of the spinal cord. b, Colorplots show the spatiotemporal maps of motor neuron activity underlying locomotion in M6 (n = 55 gait cycles), ...